Unusual presentation of xanthogranuloma of the choroid plexus

Introduction: Xanthogranulomas are rare, benign, usually asymptomatic, cutaneous tumors most frequently seen in children (juvenile xanthogranulomas). Some lesions can be found accidentally at randomly performed cerebral computer tomography (CT) or magnetic resonance imaging (MRI) or even on autopsy.Case report: We present the case of a 44 year-old woman, known with a thrombophilic disorder (PAI-1 gene mutation, MTHFR C677T and A1298C) on chronic anticoagulant treatment. The onset of symptoms was in 2010, when she presented paresthesia and lower limbs weakness. Two years later the patient presents with severe intermittent headache and left hemicrania and a cerebral angio-MRI is performed showing a left postero-inferior cerebellar artery aneurysm and two choroid plexus intraventricular masses in the lateral ventricles. The patient developed a new symptom, dysarthria in 2014 and in 2015 has multiple episodes of loss of consciousness, interpreted as epileptic seizures. Routine blood tests were within normal range, except for a high cholesterol level. The patient was tested for autoimmune, infectious, endocrine and metabolic diseases that were negative. Surgical treatment and biopsy from the lesion was proposed, however the patient refused both procedures.Conclusions: There is an association between xanthogranulomas localization and the choroid plexus, the most frequent CNS origin being in the trigon of the lateral ventricle. Our case does not resemble with any other case published, mostly because the unusual presentation, symptomatology and the association between xanthogranulomas, thrombophilia and postero-inferior cerebellar artery aneurysm which were never reported before in other cases of xanthogranulomas from the literature.

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“…Poor outcome was encountered in XG located in the third and forth ventricle, with a large number of postoperative complications. [24,25].…”

Section: Discussionmentioning

confidence: 99%

Case Presentation: Unusual Association Between Possible Bilateral Intraventricular Xanthogranulomas, Postero-inferior Cerebellar Artery Aneurysm and Thrombophilia

Andone¹,

Romaniuc²,

Bajkó

et al. 2018

Acta Medica Marisiensis

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“…The first case of a choroid plexus xanthogranuloma was reported in 1900, by Blunner as “cholesteatomatous endothelioma.” Since then, choroid plexus xanthogranulomas have been reported incidentally in up to 7% of autopsy studies, found mostly in the lateral ventricles 1 . They are more likely to become symptomatic when present in the third ventricle from obstruction of the foramen of Monro or aseptic meningitis from xanthomatous debris spilling into cerebrospinal fluid, causing hydrocephalus 3 . The fourth ventricle lesion may become symptomatic through similar mechanisms, although little information exists given how exceedingly rare they are in this location.…”

Section: Discussionmentioning

confidence: 99%

“…Some xanthogranulomas are associated with degenerative changes, including demyelination of neuronal tissues, whereas others form masses despite their nonneoplastic character. This, however, is still a matter of controversy 3 and the pathogenesis of lateral ventricular xanthogranulomas may be different 4 …”

Section: Discussionmentioning

confidence: 99%

Fourth Ventricle Choroid Plexus Xanthogranuloma Causing Hydrocephalus

Ferguson

Walling

Easton³

et al. 2015

Can. J. Neurol. Sci.

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“…Horses with solely signs of spinal cord dysfunction were not considered to have neurological signs with respect to this study. The criteria were based on the reports of cholesterinic granulomas in the veterinary literature, and those of xanthogranulomas and increased intracranial pressure in human literature . The European College of Veterinary Diagnostic Imaging resident undertook initial data collection and a European College of Veterinary Internal Medicine diplomate (A.B.S.)…”

Section: Methodsmentioning

confidence: 99%

Presumed cholesterinic granulomas detected on CT in horses are associated with increased lateral ventricle height and age

Lloyd-Edwards

Willems

Beukers

et al. 2020

Vet Radiology Ultrasound

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Cholesterinic granulomas are mass-like lesions that form at the choroid plexus of the ventricular system. Large cholesterinic granulomas within the lateral ventricles have been reported to cause severe neurological signs. However, little data are available about their prevalence or appearance in the overall population. The objective was to report the prevalence of presumed cholesterinic granulomas on CT in a population of horses, and investigate associations between presumed cholesterinic granuloma presence, lateral ventricle size, age, and neurological signs. The study was cross sectional, CT scans of the head were assessed for presumed cholesterinic granuloma presence and size, and lateral ventricle height. Computed tomography findings and clinical information were compared using nonparametric testing. Computed tomography scans of 139 horses were included. Presumed cholesterinic granulomas were found in 22 horses (15.8%), nine were unilateral and 13 bilateral. A significant increase in prevalence was observed with age (P < .0001), with 38% of horses over 15 years old affected. The median volume of presumed cholesterinic granulomas was 242 mm 3 with a range from 51 to 2420 mm 3 . The mean lateral ventricle height was significantly increased in horses with presumed cholesterinic granulomas present (P = .004), with a median of 7.3 mm compared to 4.9 mm without. Neurological signs were not associated with presumed cholesterinic granuloma presence or lateral ventricle height. Fourth ventricle mineralizations were found in seven horses, which may represent cholesterinic granulomas. In conclusion, presumed cholesterinic granulomas occurred in a large proportion of the examined population and are associated with increased lateral ventricle dilation and advanced age.

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Unusual presentation of xanthogranuloma of the choroid plexus

Cited by 6 publications

References 4 publications

Case Presentation: Unusual Association Between Possible Bilateral Intraventricular Xanthogranulomas, Postero-inferior Cerebellar Artery Aneurysm and Thrombophilia

Case Presentation: Unusual Association Between Possible Bilateral Intraventricular Xanthogranulomas, Postero-inferior Cerebellar Artery Aneurysm and Thrombophilia

Fourth Ventricle Choroid Plexus Xanthogranuloma Causing Hydrocephalus

Presumed cholesterinic granulomas detected on CT in horses are associated with increased lateral ventricle height and age

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